线粒体是控制细胞生物合成、能量代谢和信号转导的关键细胞器，通过生物发生、融合、裂变和线粒体自噬等过程维持动态平衡。越来越多的证据表明线粒体功能障碍与一系列呼吸系统疾病有关，包括急性肺损伤/急性呼吸窘迫综合征、支气管哮喘、肺纤维化、慢性阻塞性肺病和肺癌。因此，确定能够改善受损线粒体功能的方法对于治疗肺部疾病至关重要。细胞外囊泡（EV）是细胞释放到细胞外空间的纳米尺寸膜囊泡，通过在细胞或器官之间传递生物活性物质或信号来促进细胞间通讯。最近的研究在 EV 的特定亚群中发现了丰富的线粒体成分，称为线粒体细胞外囊泡 (mitoEV)，其内容和组成随疾病进展而变化。此外，mitoEVs 已在受损的受体细胞中表现出修复线粒体功能。然而，目前缺乏对 mitoEV 的全面了解，限制了其临床转化前景。本综述探讨了 mitoEV 的生物发生、分类、功能性线粒体货物和生物效应，重点关注其在肺部疾病中的作用。重点强调它们作为肺部疾病生物标志物和创新治疗策略的潜力，为各种肺部疾病的机制研究和药物开发提供新的见解。

Mitochondria, pivotal organelles governing cellular biosynthesis, energy metabolism, and signal transduction, maintain dynamic equilibrium through processes such as biogenesis, fusion, fission, and mitophagy. Growing evidence implicates mitochondrial dysfunction in a spectrum of respiratory diseases including acute lung injury/acute respiratory distress syndrome, bronchial asthma, pulmonary fibrosis, chronic obstructive pulmonary disease, and lung cancer. Consequently, identifying methods capable of ameliorating damaged mitochondrial function is crucial for the treatment of pulmonary diseases. Extracellular vesicles (EVs), nanosized membrane vesicles released by cells into the extracellular space, facilitate intercellular communication by transferring bioactive substances or signals between cells or organs. Recent studies have identified abundant mitochondrial components within specific subsets of EVs, termed mitochondrial extracellular vesicles (mitoEVs), whose contents and compositions vary with disease progression. Moreover, mitoEVs have demonstrated reparative mitochondrial functions in injured recipient cells. However, a comprehensive understanding of mitoEVs is currently lacking, limiting their clinical translation prospects. This Review explores the biogenesis, classification, functional mitochondrial cargo, and biological effects of mitoEVs, with a focus on their role in pulmonary diseases. Emphasis is placed on their potential as biological markers and innovative therapeutic strategies in pulmonary diseases, offering fresh insights for mechanistic studies and drug development in various pulmonary disorders.